Abstract

Pulmonary manifestations often occur in connective tissue diseases (CTD) affecting the different component of the respiratory system (the lung parenchyma, the airways, the pleura, the vascular system…). The frequency and the severity of these manifestations vary according to the type of manifestation and the corresponding CTD. Systematic assessment by chest high resolution computed tomography (HRCT) lead to reconsider the frequency of some pulmonary manifestations with a long asymptomatic phase such as rheumatoid arthritis (RA) associated interstitial lung disease (ILD). Among the pulmonary manifestations associated with CTD, some will progress and may lead to an important disease burden and increase mortality. If a CTD related pulmonary manifestation is detected in a patient, it may impact the therapeutic management including the prescriptions of specific treatments or the restriction of pulmonary toxic drugs. The screening for such manifestations in asymptomatic patients, which enable early deification, is therefore indicated. For systemic sclerosis, dermatomyositis and Sjögren syndrome, lung involvement screening has been established in National Diagnostic and Care Protocols (PNDS) that will be developed in this review. For other CTD, there are still no guidelines. Beside a relatively high frequency of RA-ILD, no recommendations have been developed for the screening of RA-ILD. In this review, we will give the recent data that help to discuss and argue the necessity of a screening, notably in high-risk patients.

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